Tatsumi Koichiro, Nakanishi Norifumi, Tanabe Nobuhiro, Kasahara Yasunori, Kubo Keishi, Hirai Toyohiro, Mishima Michiaki
Department of Respirology, Graduate School of Medicine, Chiba University, Chiba.
Nihon Kokyuki Gakkai Zasshi. 2010 Aug;48(8):551-64.
The Ministry of Health, Labour and Welfare (Japan) has approved research into primary pulmonary hypertension (PPH) and pulmonary hypertension due to chronic thromboembolic and/or embolic disease (CTE-PH) to examine their epidemiology, pathophysiology, and develop new therapeutic strategies. The Respiratory Failure Research Group, with grant support from the Ministry of Health, Labour and Welfare, changed the diagnostic names of PPH and CTE-PH. The Specific Diseases Control Division in the Health Service Bureau of the Ministry of Health, Labour and Welfare supported our proposal. One of the major purposes of The Respiratory Failure Research Group has been to maintain and, if possible, promote patient quality of life and prognosis in cases of intractable respiratory diseases. The name PPH has been changed to "pulmonary arterial hypertension (PAH)", and the name CTE-PH has been changed to "chronic thromboembolic pulmonary hypertension (CTEPH)", in keeping with recent worldwide research progress in this field. PAH should be subdivided into different pathophysiologic conditions, such as idiopathic and hereditary PAH, PAH associated with connective tissue diseases, portal hypertension, congenital heart disease, persistent pulmonary hypertension in newborn babies, pulmonary veno-occlusive disease etc. Different therapeutic strategies may be adopted for different subgroups. Pulmonary hypertension due to left heart disease, lung disease and/or hypoxia and CTEPH should be excluded from PAH. Continuous monitoring of PAH and CTEPH is required in patients with these conditions, even if the degree of pulmonary hypertension is improved by therapeutic intervention, because these diseases are incurable.
日本厚生劳动省已批准对原发性肺动脉高压（PPH）以及由慢性血栓栓塞和/或栓塞性疾病导致的肺动脉高压（CTE-PH）进行研究，以考察其流行病学、病理生理学并制定新的治疗策略。呼吸衰竭研究小组在厚生劳动省的资助支持下，更改了PPH和CTE-PH的诊断名称。厚生劳动省卫生局特定疾病控制司支持了我们的提议。呼吸衰竭研究小组的一个主要目标是维持并在可能的情况下改善难治性呼吸系统疾病患者的生活质量和预后。为了与该领域近期的全球研究进展保持一致，PPH的名称已更改为“肺动脉高压（PAH）”，CTE-PH的名称已更改为“慢性血栓栓塞性肺动脉高压（CTEPH）”。PAH应细分为不同的病理生理状况，例如特发性和遗传性PAH、与结缔组织病相关的PAH、门脉高压、先天性心脏病、新生儿持续性肺动脉高压、肺静脉闭塞性疾病等。针对不同亚组可能会采用不同的治疗策略。由左心疾病、肺部疾病和/或缺氧导致的肺动脉高压以及CTEPH应排除在PAH之外。即使通过治疗干预肺动脉高压程度有所改善，对于患有这些疾病的患者仍需要持续监测PAH和CTEPH，因为这些疾病无法治愈。
